So wo koma rebɔ ntɛmntɛm mpofirim? Eyi betumi ayɛ CPVT (Catecholaminergic Polymorphic Ventricular Tachycardia)!

Ɛtɔ da bi a, sɛ wobrɛ paa, anaa sɛ wote biribi kɛse bi nka wɔ wo koma mu, te sɛ anigye kɛse, awerɛhow, anaa ehu a, so wo koma bɔ ntɛmntɛm mpofirim na wo koko mu yɛ den? Ebia w’ani bɛdan bruu na wo ho ayɛ wo hyew? Mma ɛnnyɛ wo nwonwa, saa nneɛma yi betumi ato abofra ketewa mpo. Ɛnnɛ yɛrebɛka tebea soronko bi a ɛba koma no mu, a ɛho yɛ na kakra nanso ɛho hia yiye sɛ yehu ho asɛm.

Dɛn ne CPVT (Catecholaminergic Polymorphic Ventricular Tachycardia) a ɛma koma no yɛ kɛse?

Sɛ yɛbɛka no tiawa a, CPVT (Catecholaminergic Polymorphic Ventricular Tachycardia) yɛ tebea bi a ɛba wɔ koma no nnyigyei mu. Wɔ eyi mu no, wo koma no afã horow a ɛwɔ fam, kyerɛ sɛ, koma no ase, anaa sɛnea nnuruyɛfo frɛ no, ventricles , fi ase bɔ ntɛmntɛm yiye na ɛnyɛ ne kwan so mpofirim bere a woteɛteɛ w’apɔw mu, di agoru, anaa wote adwennwen pii nka, te sɛ anigye a ɛtra so, ehu, anaa anigye no.

Susuw ho saa kwan yi so: yɛn koma te sɛ nsu pɔmpɛ. Ɛyɛ nea ɛma mogya fa nipadua no nyinaa mu. Enti, sɛ koma yi bɔ ntɛmntɛm dodo, kyerɛ sɛ, wɔ nnyigyei a ɛnteɛ mu a, ɛyɛ den ma no sɛ ɛbɛbɔ mogya yiye na ɛde akɔ nipadua no afã horow nyinaa. Eyi ne nea ɛkɔ so wɔ CPVT mu titiriw.

Wɔfrɛ tebea yi CPVT abu so dɛn?

Nokwarem no, tebea a wɔfrɛ no CPVT yi ho yɛ na kakra . Sɛnea aduruyɛ ho abenfo kyerɛ no, wobu akontaa sɛ nnipa mpem du (10,000) biara mu bɛyɛ biako pɛ na wɔwɔ saa yare yi. Enti, ebia ɛnyɛ obiara na wate ho asɛm.

Dɛn nti na CPVT yi ba? Dɛn na ɛde ba?

Nnipa pii wɔ asɛmmisa bi sɛ, "Dɛn nti na eyi si?" Ade titiriw nti a CPVT ba ne nsakrae a ɛba yɛn awosu mu nkwaadɔm mu, nkwaadɔm mu nsakrae . Wunim, biribiara a ɛwɔ yɛn nipadua mu no, saa awosu mu nkwaadɔm yi na ɛhwɛ so.

• Mpɛn pii no, wubetumi anya awosu mu nsakrae yi afi wo maame anaa wo papa hɔ, anaa n’abien nyinaa hɔ.
• Nanso ɛntaa mma koraa, kyerɛ sɛ, ɛntaa nsi koraa sɛ saa awosu mu nsakrae yi betumi aba obi foforo nipadua mu kwa , a awofo no nni awosu mu nkɛntɛnso biara.

Enti, saa awosu mu nsakrae yi de ɔhaw ba ɔkwan a wɔfa so di calcium ions so wɔ koma nkwammoaa no mu no mu. Ɛno ne ade titiriw nti a koma no bɔ ntɛmntɛm bere a koma no ayɛ basaa anaasɛ bere a ɔreyɛ apɔw-mu-teɛteɛ no.

Dɛn ne CPVT ho sɛnkyerɛnne ahorow? Yɛyɛ dɛn hu no?

CPVT ho sɛnkyerɛnne taa fi ase wɔ mmofraase, mpɛn pii no efi mfe 7 kosi 12. Mpɛn pii no, sɛnkyerɛnne ahorow no di kan da adi bere a watu mmirika na wadi agoru pii akyi , anaasɛ bere a obi nya nkate a emu yɛ den , te sɛ anigye, ehu, abufuw, anaa anigye.

Nneɛma bi a ebetumi ayɛ CPVT ho sɛnkyerɛnne ni:

• Ɛsen onipa mpapahwekwaEbia wotaa totɔ piti, anaasɛ wobɛte nka sɛ wo ti ayɛ wo yaw anaasɛ wo ti yɛ hare. Ɛtɔ mmere bi a, wubetumi ahwere w’adwene mpofirim a ntease biara nnim na woahwe ase.
• Koma a ɛbɔ yɛ mpofirim, ntɛmntɛm yiye, a ɛbɔ a ɛte sɛ nea obi retu mmirika akɔ wo moma so.
• Ebia nnipa binom mpo behyia tebea horow te sɛ akisikuru . Eyi ba efisɛ mogya dodow a ɛkɔ amemene no mu no so tew bere a koma no ntumi nkɔ mogya no yiye no.

Nea ɛho hia: Sɛ wo ba no ho popo mpofirim bere a ɔredi agoru, ɔretu mmirika, anaa ɔrehuruw, anaasɛ onwiinwii sɛ ɛyɛ den sɛ ɔbɛhome a, mmu w’ani ngu so kɛkɛ. Ɛho hia sɛ wohwehwɛ aduruyɛfo afotu ntɛm ara.

Ɔkwan bɛn so na oduruyɛfo hu CPVT pɛpɛɛpɛ?

Sɛ wo anaa wo ba nya saa sɛnkyerɛnne yi a, oduruyɛfo bɛyɛ nhwehwɛmu pii de akyerɛ sɛ wowɔ CPVT anaa.

• Echocardiogram - echo test: Eyi te sɛ koma no mu ultrasound scan. Ɛde nnyigyei asorɔkye di dwuma de hu sɛnea wo koma te, sɛnea koma no afasu reyɛ adwuma yiye, sɛ ebia ntini no reyɛ adwuma yiye, ne sɛnea mogya retu no pefee.
• ECG (Electrocardiogram): Saa sɔhwɛ yi fa wo koma no anyinam ahoɔden nsɛnkyerɛnne, anaa koma no nnyigyei ho mfonini. Ebia obi a ɔwɔ CPVT renkyerɛ nsakrae titiriw biara wɔ ECG so bere a ɔwɔ tebea a ɛfata mu no.
• Apɔw-mu-teɛteɛ mu Adwennwen Sɔhwɛ: Eyi yɛ sɔhwɛ a ɛho hia yiye a wɔde hu CPVT. Bere a woretu mmirika wɔ treadmill so anaasɛ wote sakre so no, wɔkɔ so hwɛ wo koma bɔ ne ECG so. Ɛda adi sɛ nnipa a wɔwɔ CPVT no koma bɔ ntɛmntɛm wɔ ɔkwan a ɛnteɛ so wɔ saa apɔw-mu-teɛteɛ yi mu.
• Mfoniniyɛ mu nhwehwɛmu afoforo: Wɔ tebea horow bi mu no, ebia wo duruyɛfo bɛkamfo akyerɛ sɛ ɔnhwɛ wo koma no mu kɔ akyiri, te sɛ MRI (Magnetic Resonance Imaging ), koma mu ntini a wɔde hyɛ wo koma mu , anaa koma mu ntini a wɔde hwɛ wo koma mu .
• Awosu mu Nhwehwɛmu: Saa nhwehwɛmu ahorow yi hwehwɛ awosu mu nsakrae pɔtee bi (nsakrae mu nsakrae) a ɛde CPVT ba mogya mu. Eyi betumi asi yare no so dua na ahu sɛ abusua no mufo afoforo wɔ asiane mu anaa.

Ɛtɔ da bi a, wo duruyɛfo betumi ama wo afiri ketewaa bi a wubetumi de ahyɛ mu nna kakraa bi anaa adapɛn kakraa bi. Wɔfrɛ eyi sɛ ambulatory monitor anaa Holter monitor.Ɛkyerɛw anyinam ahoɔden dwumadi a ɛwɔ wo koma mu da mũ no nyinaa (24, 48, anaa nea ɛboro saa mpo) bere a woyɛ wo da biara da dwumadi ahorow na woyɛ wo fie nnwuma a wotaa yɛ no. Eyi ma wo duruyɛfo nya sɛnea wo koma rebɔ ho adwene pa, bere mpo a wunni sɛnkyerɛnne biara no.

Dɛn ne ayaresa ahorow a wɔde sa CPVT?

Sɛ koma no fi ase bɔ ntɛmntɛm dodo mpofirim, kyerɛ sɛ, sɛ ɛkɔ tebea a ɛne ventricular tachycardia (Ventricular Tachycardia) mu a, ebetumi de nkwa ato asiane mu. Wɔ tebea horow a ɛtete saa mu no, ebia ɛho behia sɛ wɔde anyinam ahoɔden bɔ (Cardioversion anaa Defibrillation) na ama koma no asan ayɛ nea ɛfata. Wɔtaa yɛ eyi sɛ ayaresa a egye ntɛmpɛ.

Nanso bere tenten mu no, botae titiriw a ɛwɔ CPVT a wɔsa mu ne sɛ wɔbɛma koma no nnyigyei anyɛ ntɛmntɛm wɔ ɔkwan a ɛnteɛ so na wɔakɔ so ayɛ nea ɛfata. Ebia nea edidi so yi mu biako anaa nea ɛboro saa bɛka wo ayaresa nhyehyɛe no ho:

• Nnuru a wɔde yɛ:
• Beta-blockers: (e.g. Lopressor®, Corgard®) Saa nnuru yi yɛ adwuma denam nkɛntɛnso a hormone te sɛ adrenaline nya wɔ koma no so no so. Eyi boa ma wodi koma a ɛbɔ no so na ɛnkɔ soro bere a wɔreyɛ apɔw-mu-teɛteɛ anaa adwennwen no. Eyi ne nnuru titiriw a wɔde ma CPVT.
• Calcium Channel Blockers: (e.g. Cardizem®, Procardia®) Ɛtɔ da bi a wobetumi de saa aduru yi aka beta-blockers ho.
• Nnuru afoforo a wɔde siw komayare ano: Wobetumi ama nnipa binom nnuru afoforo te sɛ flecainide.
• Implantable Cardioverter Defibrillator (ICD): Eyi yɛ afiri ketewaa bi a wɔde batere na ɛyɛ adwuma a wɔde oprehyɛn hyɛ koko ho were ase. Ɛkɔ so hwɛ sɛnea koma no bɔ denneennen. Sɛ koma no nnyigyei yɛ nea ɛnkɔ so pɛpɛɛpɛ mpofirim wɔ ɔkwan a asiane wom so a, afiri no ankasa de anyinam ahoɔden ma de san ba. Nnuruyɛfo taa kamfo ICD kyerɛ nnipa a asiane kɛse wom na ɛyɛ den sɛ wɔde nnuru nkutoo bedi wɔn koma a ɛyɛ den no so.
• Left Cardiac Sympathetic Denervation (LCSD): Eyi yɛ oprehyɛn a woyi ntini a ɛkɔ koma no mu no bi fi hɔ. Eyi betumi atew koma no nkanyan so na atew asiane a ɛwɔ hɔ sɛ nnyigyei a ɛyɛ hu bɛba no so. Wobetumi asusuw oprehyɛn yi ho ama nnipa a wɔwɔ ICD a wɔtaa ho dwiriw wɔn anaasɛ wɔnyɛ wɔn ade yiye wɔ nnuru ho.

Nea ɛka ho no, ebehia sɛ woyɛ nsakrae ahorow bi a ɛho hia wɔ w’asetra kwan mu .

• Ɛsɛ sɛ wɔto apɔw-mu-teɛteɛ a emu yɛ den , te sɛ mmirikatu a wɔde tu mmirika ntɛmntɛm anaa nneɛma a emu yɛ duru a wɔma so no ano hye.
• Wɔ abofra kumaa a ɔwɔ CPVT fam no, eye sɛ ɔbɛtwe ne ho afi akansi agumadi ho , efisɛ anigye ne nhyɛso a ɛwɔ akansi bi mu nkonimdi mu no betumi ama koma no abɔ ntɛmntɛm.
• Ɛho hia paa nso sɛ wosua akwan a wobɛfa so atew adwennwen so.

So yebetumi asiw CPVT yi ano sɛ ɛremma?

Esiane sɛ awosu mu nsakrae na ɛde CPVT ba nti, ɔkwan biara nni hɔ ankasa a wɔbɛfa so asiw ano koraa sɛ ɛremma . Efisɛ ɛnyɛ biribi a yebetumi adi so.

Nanso, sɛ wohu CPVT wie a, sɛ wɔhwɛ so yiye, kyerɛ sɛ, sɛ wɔde nnuru a oduruyɛfo no akyerɛw ama no di dwuma yiye a, wɔyɛ asetra mu nsakrae sɛnea wɔakyerɛw ama wɔn no, na wɔkɔ oduruyɛfo nkyɛn daa a, obi a ɔwɔ CPVT mpo betumi atra ase wɔ ɔkwan a ɛfata so kɛse, na ɛyɛ papa.

Sɛ wɔahu sɛ obi a ɔwɔ w’abusua mu anya CPVT a, ɛho hia sɛ wususuw awosu mu nhwehwɛmu ne awosu mu afotu a wɔde bɛma abusua no mufo afoforo, titiriw mmofra ho. Awosu ho ɔfotufo betumi aboa wo ma woate tebea no ase yiye, aka hokwan a ɛwɔ hɔ sɛ wo mma benya bi afi awo mu ho asɛm, na woasusuw nsɛm a ɛho hia te sɛ nea wubetumi ayɛ wɔ ho no ho.

Dɛn na wobetumi ayɛ na wɔakwati nsɛnnennen a ebetumi aba esiane CPVT nti?

Sɛ wɔanhwɛ CPVT so yiye a, nsɛnnennen bi a asiane wom betumi aba. Wubetumi ayɛ saa nneɛma yi de atew asiane a ɛwɔ hɔ no so:

• Wo ne wo duruyɛfo nkasa na yɛ apɔw-mu-teɛteɛ nhyehyɛe a ahobammɔ wom a ɛbɛyɛ adwuma ama wo. Ɛnsɛ sɛ wugyae apɔw-mu-teɛteɛ koraa, nanso ɛsɛ sɛ wuhu apɔw-mu-teɛteɛ a eye ne nea ɛnyɛ papa.
• To anonne a caffeine wom te sɛ kɔfe, tii, chocolate bi, ne anonne a ɛyɛ dɛ bi ano hye sɛnea wubetumi, efisɛ caffeine yɛ ade a ɛkanyan wo na ebetumi ama wo koma abɔ kɛse.
• Hwɛ wo yare no ho sɛnkyerɛnne ahorow so bere nyinaa . Sɛ wote nka sɛ wo ho ayɛ wo yaw, wo koko mu ayɛ wo den, anaasɛ ɛyɛ den sɛ wobɛhome a, mmu w’ani ngu saa sɛnkyerɛnne ahorow yi mu biara so. Frɛ wo duruyɛfo ntɛm ara.
• Mma da koro nnuru a wo duruyɛfo akyerɛw ama wo no nmpa wo ti so da, fa no bere a ɛfata ne dodow a ɛfata mu .
• Ɛho hia paa sɛ wobɛkɔ oduruyɛfo nkyɛn bere ano na woayɛ nhwehwɛmu a ɛho hia. Eyi yɛ biribi a ɛsɛ sɛ woyɛ wɔ w’asetra nyinaa mu.

Daakye bɛn na obi a ɔwɔ CPVT betumi ahwɛ kwan?

Sɛ wɔansa CPVT a, ebetumi de akwahosan ho haw ne nsɛnnennen aba. Nsɛnnennen bi, te sɛ koma a ɛtɔ mpofirim , betumi de nkwa ato asiane mu.

Nanso, asɛmpa no ne sɛ, sɛ wɔsa wɔn yare yiye na wonya asetra pa a, nnipa dodow no ara a wɔwɔ CPVT no betumi akura koma a ɛyɛ den mu, atew asiane a ɛwɔ hɔ sɛ wobenya saa nsɛnnennen yi so kɛse, na wɔatra ase anigye ne abotɔyam.

Sɛ wowɔ CPVT a, wubehia ɔhwɛ a wodi akyi wɔ wo nkwa nna nyinaa mu na ama woahwɛ ahu sɛ wo koma no yɛ nea ɛfata.Ɛho hia. Ɛno kyerɛ sɛ ɛsɛ sɛ wokɔ oduruyɛfo nkyɛn daa na woyɛ nhwehwɛmu a ɛho hia. Sɛ CPVT ho sɛnkyerɛnne san ba bio, te sɛ nea wote nka sɛ wo ho yɛ wo yaw bere a woateɛteɛ w’apɔw mu awie no a, kɔ wo duruyɛfo nkyɛn ntɛm ara a wontwentwɛn wo nan ase.

Nneɛma a ɛsɛ sɛ wubisa wo duruyɛfo

Sɛ wo anaa wo ba wɔ saa sɛnkyerɛnne ahorow yi, anaasɛ wɔahu sɛ wowɔ CPVT dedaw a, ɛbɛyɛ mmoa kɛse sɛ wubebisa wo duruyɛfo no pefee wɔ nea edidi so yi ho:

• Dɛn na ɛda adi sɛ ɛde saa sɛnkyerɛnne ahorow yi ba? So eyi yɛ CPVT?
• Nhwehwɛmu afoforo bɛn na ɛsɛ sɛ me anaa me ba no yɛ de si so dua sɛ mewɔ CPVT anaa?
• Dɛn ne ayaresa a eye sen biara a wobetumi apaw ama CPVT? Ayaresa bɛn na eye ma me/me ba?
• Mfaso ne nsunsuanso bɔne bɛn na ɛwɔ saa ayaresa ahorow yi so?
• Dɛn na ɛsɛ sɛ woyɛ wɔ oduruyɛfo nsrahwɛ ntam, na ama woadi yare no ho sɛnkyerɛnne ahorow ho dwuma wɔ fie, anaasɛ bere a egye ntɛmpɛ?
• Nsakrae bɛn na ɛsɛ sɛ meyɛ wɔ m’asetra kwan mu (aduan, apɔw-mu-teɛteɛ)?
• So abusua no mufo afoforo wɔ asiane mu? So ɛsɛ sɛ wɔsɔ wɔn nso hwɛ?

Awiei koraa no, nea ɛsɛ sɛ wokae (Take-Home Message) .

Enti, ɛwom sɛ tebea a yɛaka ho asɛm tenten, CPVT (Catecholaminergic Polymorphic Ventricular Tachycardia), betumi ayɛ te sɛ nea ɛyɛ hu kakra de, nanso ade a ɛho hia sen biara ne sɛ wubehu ntɛm na woanya ayaresa yiye.

Sɛ wohu yare no yiye, sa wo yare sɛnea wo duruyɛfo kyerɛ wo, na wosakra asetra mu nsakrae nketenkete bi a, obi a ɔwɔ CPVT mpo betumi atra ase wɔ ɔkwan a ɛfata so, ɔyɛ nnam, na abotɔyam wom kɛse. Kae sɛ, sɛ wowɔ yare no ho sɛnkyerɛnne bi a, ɛho hia sɛ wokɔ oduruyɛfo nkyɛn ntɛm ara a wummu w’ani ngu so. Ɛnyɛ wo nkutoo na wowɔ akwantu yi mu, na nnuruyɛfo ne akwahosan ho adwumayɛfo a wɔn ho akokwaw wɔ hɔ a wobetumi aboa wo na wɔahwɛ wo. Enti nsuro, fa akokoduru gyina eyi ano.

` CPVT, koma a ɛbɔ, koma bɔ, ventricular tachycardia, awosu mu nsakrae, ɔyare a ɛma obi hwe ase, koma a ɛtwetwe mpofirim, mmofra akwahosan